title: [Attainment of complete hematological remission is crucial for extended survival of AL amyloidosis patients with cardiac involvement].
abstract: BACKGROUND
  Symptomatic cardiac involvement is the most important prognostic factor in AL amyloidosis patients. Longterm survival is limited not only by cardiac involvement condition, but also by limited choice of treatment with unsatisfactory results. The aim of the present report is to assess the effect of achieved treatment response on survival of AL amyloidosis patients with symptomatic cardiac involvement under conventional treatment.
  
  
  METHODS
  The monitored patient set consisted of 19 patients with systemic AL amyloidosis and symptomatic cardiac involvement, treated and monitored at the III. Clinic of Internal Medicine between 2004 and 2012. The male : female ratio was 17 : 2, and the age median was 64 (range 48 to 78 years). Thirteen patients died within the monitored period. Functional status was defined according to the NYHA classification, where five patients had class II involvement, 10 patients had class III involvement, and four patients had class IV involvement. Treatment response was assessed by the application of modified IMWG and ISA criteria; all patients were undergoing conventional treatment. Nine patients were treated by a combination of alkylating agents (alkeran, cyclophosphamide), six were treated by a combination treatment with thalidomide, and four were treated by a combination of bortezomib and dexamethasone. Data were analyzed with software SPSS v. 15 (SPSS, Inc., Chicago, USA). Log Rank Test was applied to survival evaluation.
  
  
  RESULTS
  The statistical analysis included only 13 patients who underwent at least three months of treatment, where six patients attained complete remission (CR), four patients attained partial remission (PR), and three patients attained only stabilization of disease (SD). Significant difference in patient survival was found to be correlated with attained hematological response, where the patients who attained CR had median survival of 39 months vs 10 months in patients who attained PR or SD (p = 0.005).
  
  
  CONCLUSIONS
  The results indicate that attainment of complete hematological remission is associated with significantly longer survival of AL amyloidosis patients with symptomatic cardiac involvement.
authors.forename: T
authors.initials: T
authors.lastname: Pika
authors.forename: P
authors.initials: P
authors.lastname: Lochman
authors.forename: J
authors.initials: J
authors.lastname: Vymětal
authors.forename: R
authors.initials: R
authors.lastname: Metelka
authors.forename: J
authors.initials: J
authors.lastname: Minařík
authors.forename: P
authors.initials: P
authors.lastname: Látalová
authors.forename: J
authors.initials: J
authors.lastname: Zapletalová
authors.forename: J
authors.initials: J
authors.lastname: Bačovský
authors.forename: V
authors.initials: V
authors.lastname: Ščudla
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000150:complications / Q000188:drug therapy* / Q000401:mortality; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001897:Boronic Acids / Q000008:administration & dosage; D000069286:Bortezomib; D003520:Cyclophosphamide / Q000008:administration & dosage; D003907:Dexamethasone / Q000008:administration & dosage*; D005260:Female; D006338:Heart Neoplasms / Q000150:complications / Q000188:drug therapy* / Q000401:mortality; D006801:Humans; D000075363:Immunoglobulin Light-chain Amyloidosis; D008297:Male; D008558:Melphalan / Q000008:administration & dosage; D008875:Middle Aged; D011719:Pyrazines / Q000008:administration & dosage; D012074:Remission Induction / Q000379:methods*; D013792:Thalidomide / Q000008:administration & dosage; D016896:Treatment Outcome
chemical_list: D001897:Boronic Acids; D011719:Pyrazines; D013792:Thalidomide; D000069286:Bortezomib; D003907:Dexamethasone; D003520:Cyclophosphamide; D008558:Melphalan
keywords: amyloidosis - cardiomyopathy - treatment - survival